Abstract
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder belonging to the group of transmissible spongiform encephalopathies. Transition of physiological, soluble, neuroprotective prion protein PrPc into its insoluble, misfolded isoform PrPSc remains the central pathogenic event in this disorder. Their progressive accumulation within the brain tissue results in spongiform degeneration and plethora of clinical symptoms. Typically, CJD manifests as progressive dementia with myoclonus, visual or cerebellar dysfunction, pyramidal/extrapyramidal signs or akinetic mutism. However, growing number of studies indicate that CJD may present with prodromal psychiatric manifestations including anhedonia, anxiety, irritability, depression, insomnia, psychosis and catatonic behaviour. We present case of biopsy proven CJD with a history of prodromal psychiatric symptoms and catatonic behaviour.
Highlights
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder belonging to the group of transmissible spongiform encephalopathies
A growing number of studies indicate that CJD may present with prodromal psychiatric manifestations including anhedonia, anxiety, irritability, depression, insomnia, psychosis and behavioral changes [3]
All of these reported cases indicate that CJD may manifest with a plethora of uncharacteristic, atypical psychiatric symptoms and it is likely that psychiatrists may be the first-line doctors dealing with a CJD patient at the early stages of the disease [11]
Summary
Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disorder belonging to the group of transmissible spongiform encephalopathies. Siwek et al [10] presented a case of a 34-year-old female with CJD, primarily diagnosed with conversion disorder, who manifested with blurred vision, diplopia and gait impairment All of these reported cases indicate that CJD may manifest with a plethora of uncharacteristic, atypical psychiatric symptoms and it is likely that psychiatrists may be the first-line doctors dealing with a CJD patient at the early stages of the disease [11]. We present a case of CJD diagnosed by brain biopsy in a patient with a history of prodromal psychiatric symptoms and catatonic behavior. Because of the lack of typical CJD findings from the EEG and CSF examination and a concomitant clinical and MRI picture suggesting diffuse encephalopathy, brain biopsy was performed after obtaining the approval from a family court. These results confirmed the diagnosis of CJD in our patient
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